Takayasu's arteritis (TA) is a granulomatous vasculitis of medium and large arteries, which most often presents as pulseless disease due to widespread arterial stenoses. Only the minority of TA patients have aortic valve insufficiency, which is due to aortic root dilatation following aortitis and aneurysm formation. No other cardiac valve is involved. We report a case of Takayasu's disease-related severe aortic insufficiency (AI) in a Filipino woman, which necessitated aortic valve replacement. It is important to consider TA in the differential diagnosis of AI in young women, particularly those with early-onset systemic hypertension and pulse deficits. Early diagnosis and therapy of TA can improve outcomes.